FA11 (light chain, Cleaved-Ile388) rabbit pAb

catalytic activity:Selective cleavage of Arg-|-Ala and Arg-|-Val bonds in factor IX to form factor IXa.,disease:Defects in F11 are the cause of F11 deficiency [MIM:612416]; also called plasma thromboplastin antecedent deficiency or Rosenthal syndrome. It is a blood coagulation abnormality occurring in high frequency in Ashkenazi jews. F11-deficient patients are prone to excessive bleeding after haemostatic challenge.,function:Factor XI triggers the middle phase of the intrinsic pathway of blood coagulation by activating factor IX.,online information:Factor XI entry,PTM:Activated by factor XIIa (or XII), which cleaves each polypeptide after Arg-387 into the light chain, which contains the active site, and the heavy chain, which associates with high molecular weight (HMW) kininogen.,similarity:Belongs to the peptidase S1 family. Plasma kallikrein subfamily.,similarity:Contains 1 peptidase S1 domain.,similarity:Contains 4 apple domains.,subunit:Homodimer; disulfide-linked. After activation the heavy and light chains are also linked by a disulfide bond.,tissue specificity:Isoform 2 is produced by platelets and megakaryocytes but absent from other blood cells.,